Disseminated aspergillosis in a patient with cystic fibrosis and allergic bronchopulmonary aspergillosis.

Allergic bronchopulmonary aspergillosis (ABPA) is a well-known complication of cystic fibrosis (CF), with an estimated incidence of up to 11%. In patients with CF, the diagnosis of ABPA must be based on significant elevation of Aspergillus fumigatus (Af) antibody and total serum IgE, since it is common to already have other clinical and laboratory features of ABPA (Laufer et al., J Allergy Clin Immunol. 1984; 73:44-48). Once ABPA is identified in a patient, institution of corticosteroids is standard therapy. Invasive aspergillosis is a rare occurrence in patients with CF with or without ABPA, and has been reported in only one young adult who did not have ABPA (Giudotti et al., Am J Med Sci. 1982; 283:157-160). We present a case of a 15-year-old male with CF and ABPA who developed disseminated aspergillosis.

Button gastrostomy obstructing the ileocecal valve removed by colonoscopic retrieval.

A 16-year-old male with profound developmental disability acutely developed retching, irritability, and abdominal distension. He had undergone a fundoplication and placement of a feeding gastrostomy 6 months earlier. A button gastrostomy tube was subsequently placed, but was noted to be missing from the stoma when the patient was awakened by his mother one morning. Within 48 hours he developed signs of abdominal obstruction. Abdominal radiography confirmed the presence of the gastrostomy button at the ileocecal valve. After a trial of decompression and medical management failed, the button was successfully removed endoscopically, resulting in prompt resolution of the obstruction. Gastrostomy buttons are a newer alternative to conventional catheters, but can become dislodged and migrate, as illustrated by this case. Refinements in the outer wing design may be needed.

Isoenzyme studies in transient hyperphosphatasemia of infancy. Ten new cases and a review of the literature.

A literature review and ten new cases of benign transient hyperphosphatasemia of infancy are presented, with special attention paid to isoenzyme studies. Polyacrylamide gel electrophoresis, heat denaturation, and binding of alkaline phosphatase to anti-human alkaline phosphatases showed that the sources of the elevated alkaline phosphatase levels are normal bone and liver and not the small intestine. The data also suggest that the following criteria be present for a diagnosis of transient hyperphosphatasemia: (1) an age of less than 5 years, (2) variable symptoms, (3) no bone or liver disease noted on physical examination or (4) from laboratory investigations, (5) isoenzyme analysis showing elevations in both bone and liver activity, and (6) a return to normal serum alkaline phosphatase activity values within four months.

Distal ileostomy drip feedings in neonates with short bowel syndrome.

When a newborn infant presents with high intestinal atresia, the proximal segment of the bowel is usually grossly distended and atonic. The anastomosis of this segment to the smaller and unused distal segment will usually result in little or no propulsion of contents distally. Many techniques have been employed to correct this problem. A common surgical approach is immediate end-to-end anastomosis, followed by parenteral alimentation until return of normal function. This can take many weeks, and requires special attention to fluid loss and complications associated with parenteral alimentation. In this paper we report two infants in whom we utilized a new technique to circumvent these problems. The technique involves continuous drip ileostomy feedings through the distal ileostomy, while basic nutritional needs are being met parenterally. In addition, the secretions from the proximal stoma are collected and infused with the elemental feeding. The distal bowel, now being fully utilized, is stimulated to accommodate, and when the two ends are joined at a second operation, nearly normal anatomical bowel is present.

Fatal fulminant hepatitis with hemolysis in Wilson's disease. Criteria for diagnosis.

We report a child with the presentation of Wilson's disease as acute fulminant hepatic failure and severe hemolysis. Our review of the literature suggests the following criteria for considering this diagnosis in the child with acute liver failure: discordance between mildly elevated serum transaminases and extremely elevated bilirubin levels; anemia associated with hemolysis (increased reticulocytes in the absence of bleeding); elevated hepatic copper; and other copper metabolic abnormalities (elevated serum copper, excessive 24-hour urine copper excretion, and reduced serum ceruloplasmin). Establishing the correct diagnosis enhances the possibility of detecting asymptomatic siblings or other family members in whom early inauguration preventive therapy should be successful.

Menetrier's disease in childhood: report of two cases and a review of the literature.

Two new cases plus 13 previously reported cases of Menetrier's disease in childhood are reviewed. The most common features of this illness are severe upper gastrointestinal symptoms (abdominal pain, nausea, vomiting, hematemesis) followed by the onset of generalized edema and ascites. Significant laboratory findings include hypoalbuminemia, eosinophilia, and anemia. Gastrointestinal protein loss and decreased gastric acid secretion can be documented. Upper gastrointestinal radiographs demonstrate the characteristic hypertrophic gastric rugae. Histologic features include hypertrophic tortuous gastric glands, basilar cysts, and interstitial round cell inflammation. The natural course of this disease in childhood is usually benign and self-limited. Uncommonly, the course may be severe and require gastric resection. This is in contrast to the adult form, where chronicity and severity is the rule. The etiology of this problem remains unknown. Endoscopy and biopsy are the diagnostic procedures of choice, although laparotomy may be necessary in equivocal cases. Therapy should be supportive except for those few patients who require surgery to control hemorrhage.

The 1-hr blood xylose test in the evaluation of malabsorption in infants and children.

One hundred ninety-seven 1-hr blood xylose absorption tests with 5 g of D-xylose were performed on 171 infants and children for the evaluation of malabsorption. The mean +/- 1 SD blood xylose level in 78 controls was 37.0 +/- 7.7 mg/dl, and 71 patients with post infectious diarrhea was 28.4 +/- 11.0 mg/dl. A significant reduction (P < 0.001) was found in 10 patients with Celiac disease (12.0 +/- 8.6 mg/dl) and 18 patients with intractable diarrhea syndromes (18.3 +/- 12.5 mg/dl). Forty-four patients had a blood xylose level less than 20 mg/dl, and the majority (35/44) were in the groups with postinfection and intractable diarrhea syndromes. Multiple tests were performed on 20 patients. Sixty-two intestinal biopsies were correlated with 1-hr blood xylose levels. The rate of false positives was 0. The rate of false negative tests varied between 4 to 32.7% depending on the assessment of intestinal damage. Our results confirm that the 1-hr blood xylose absorption test is a sensitive indicator of severe intestinal damage. Intestinal biopsy should be considered in all patients with a 1-hr blood xylose level less than 20 mg/dl. With borderline results (20 to 25 mg/dl), clinical decisions must be individualized.